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Year : 2017 | Volume : 5 | Issue : 2 | Page : 80 - 81  


Case Reports
Idiopathic Clubbing: A Case Report

NS Neki 1, Gagandeep Singh Shergill 2, Amritpal Singh 3, Amanpreet Kaur 4, Taranjeet Singh 5

1 Professor, 2 Post Graduate student, 3 Senior Resident, Department of General Medicine, Government Medical College & Guru Nanak Dev Hospital, Amritsar, India

4 Consultant Gynaecologist, Civil Hospital Fatehgarh Sahib, Fatehgarh Sahib, Punjab, India

5 Registrar, Department Of Oncology, Atremis Hospital, Gurgaon, Haryana, India

Corresponding Author:

Dr. N. S. Neki

Email- drneki123@gmail.com

ABSTRACT:

Clubbing of the fingers and toes has been recognized as a clinical manifestation of intra thoracic disease from the earliest times. Hippocrates particularly described the condition as occurring with advanced phthisis and empyema and emphasized the importance of the changes as diagnostic of purulent pleural effusion. Many subsequent authors have described clubbing of the fingers associated with chronic disease of the heart or lungs, but it is still rare in medical literature to find the condition mentioned as being of primary origin. We here present the case of 24 years old female having developed clubbing of all fingers that had no found secondary cause after an extensive search and so thought to be of primary origin.

Key words: Clubbing; Primary; Hypertrophic osteoarthropathy

INTRODUCTION:

Although finger clubbing is relatively uncommon but if present it is usually associated with significant underlying disease. The major conditions associated with clubbing and hypertrophic osteoarthropathy are lung diseases (75-80%), cardiovascular diseases (10-15%), diseases of liver & gastrointestinal tract (5-15%) and miscellaneous causes (5-15%). 1 Rarely finger clubbing may be idiopathic or primary occurring in the absence of apparent known cause.

CASE REPORT:

A 64 years old female, retired government employee in the education department presented with painless bulbous swelling of all finger & toes since 8 months. The swelling was gradually progressive. She had no H/o chest pain, syncope, palpitation, cyanosis, ankle swelling or any gastrointestinal complaints. There was no past history of any major illness. She had no family history of clubbing. On examination, pulse rate was 80/- min, regular; BP was 130/80 mmHg; and respiratory rate was 18/min. There was no evidence of cyanosis, pallor, jaundice or lymphadenopathy. Examination of hands & feet revealed drumstick type of clubbing. There was no swelling or tenderness of the wrists, elbows, ankles or knees. Also there was no thickening of the skin over arms or legs. On investigations, her blood counts, biochemical parameters thyroid, renal & liver profile all were normal. X-ray chest, ECG, Spirometry, 2D Echocardigraphy and CT scan chest were normal RA factor and anti ds DNA were within normal limits. X-ray of the hands & wrist did not reveal any evidence of periostitis or new bone formation. In view of the above findings, a diagnosis of idiopathic clubbing was thought since cause of clubbing could not be ascertained

DISCUSSION:

Idiopathic or primary clubbing is a rare phenomenon. 1, 2 It is of 2 types (a) Hereditary or familial forms (b) Associated with pachy-dermoperiostosis. The hereditary clubbing develops during childhood in the absence of any associated disorder, persists throughout life & is autosomal dominant3. Clubbing associated with hypertrophic osteoarthropathy, also known as pachy-dermoperiostosis or Touraine–Solente–Gole syndrome is associated with clubbing, periostitis and skin changes. Finger clubbing is a deformity of the fingers in which focal and bulbous swelling of the distal phalanges is accompanied by changes to the angles of the nail bed. This important clinical sign is associated with a number of diseases involving multiple organ systems. Lung is the most common cause of clubbing. clubbing often occurs in heart and lung diseases that reduce the amount of oxygen in the blood, such as, heart defects that are present at birth (congenital); chronic lung infections that occur in people with bronchiectasis, cystic fibrosis, or lung abscess; infection of the lining of the heart chambers and heart valves (infectious endocarditis), which can be caused by bacteria, fungi or other infectious substances; lung disorders in which the deep lung tissues become swollen and then scarred (interstitial lung disease).Other causes of clubbing include celiac, cirrhosis of the liver and other liver diseases, dysentery, graves disease, overactive thyroid gland, other types of cancer, including liver, gastrointestinal, Hodgkin’s lymphoma& rarely it may be familial also. Finger clubbing also may occur, without evident underlying disease, as an idiopathic form or as a Mandelion dominant trait4.Alterations in the size and configuration of the clubbed digit result from changes in the nail bed, beginning with increased interstitial edema early in the process. As clubbing progresses, the volume of the terminal portion of the digit may increase because of an increase in the vascular connective tissue and change in quality of the vascular connective tissue, although some cases have been associated with spurs of bone on the terminal phalanx. Although clubbing is a common physical finding in many underlying pathological processes, surprisingly, the mechanism of clubbing remains unclear. Different pathological processes may follow different pathways to a common end. Many studies have shown increased blood flow in the clubbed portion of the finger. Most researchers agree that this results from an increase in distal digital vasodilation, the cause of which is unknown. Also unknown is the exact mechanism by which increased blood flow results in changes in the vascular connective tissue under the nail bed. Whether the vasodilation results from a circulating or local vasodilator, neural mechanism, response to hypoxemia, genetic predisposition, or a combination of these or other mediators is not agreed on currently. Proposed systemic vasodilatory factors include ferritin, prostaglandins, bradykinin, adenine nucleotides, and 5-hydroxytryptamine which are thought to be escaped from metabolism in the lung in right to left shunt diseases or thought to be produced from the lung in case of diseases of the lungs. Genetic inheritance and predisposition also may play a role in digital clubbing. Hereditary clubbing is observed in 2 forms, including idiopathic hereditary clubbing and clubbing associated with pachy-dermoperiostosis. The 2 forms are believed to be separate entities. Both demonstrate autosomal dominant inheritance with incomplete penetrance. According to the most recent theory, platelet-derived growth factor released from fragments of platelet clumps or megakaryocytes has been proposed as the mechanism by which digital clubbing occurs. Megakaryocyte and platelet clumps are impacted in the digital vessels release platelet derived growth factor (PDGF) and vascular endothelial growth factor (VEGF); both are hypoxically regulated local vasodilators5. The hypoxic environment created when the capillaries become occluded, which further promotes VEGF and PDGF release from vascular endothelial cells, along with a number of other hypoxia inducible factors (HIFs)6.PDGF promotes growth, vascular permeability and chemo taxis, explaining the increased vascular smooth muscle cells and fibroblasts seen in clubbed fingers7. VEGF promotes neovascularisation and connective tissue changes, and is also known to increase vascular permeability leading to oedema8. In Belgian study researchers found that approximately 1% demonstrated signs of finger clubbing. Of those patients with finger clubbing, 40% were eventually found to have serious underlying disease over the one year follow up period, while in the remaining 60% cause of clubbing was not found9. We presumed that clubbing in this patient was due to congenital variety but it was unlikely in view of the fat that she had undergone medical check up at the time of recruitment into government service. The patient also delivered male baby one year ago and this gross clubbing could not have been missed during that medical consultation also. So it is definitely a case of idiopathic clubbing which is a rare occurrence. The patient is on regular follow up since then with no fresh complaints.

CONCLUSION:

Clubbing is an important clinical sign and is associated with a number of diseases involving multiple organ systems. First described by Hippocrates in 400BC, the underlying pathophysiology and clinical significance of finger clubbing are still subject to debate, as is the best way to examine the fingers. Although finger clubbing is relatively innocuous, yet it is important because of its frequent association with significant underlying serious cardiac or pulmonary diseases or diseases of the liver and gastrointestinal tract. Finger clubbing may also occur rarely without evidence of underlying disease, in an idiopathic or primary form. Given the current gaps in our knowledge, how effectively can we use the discovery of finger clubbing to guide us depends upon the clinical scenario.

REFERENCES:

  1. Friedman HH. Clubbing In: Problem – Oriented Medical Diagnosis. Friedman MH (Ed) VIItheduLipponpott Williams and Wilkins: Philadelphia, 2001: 277-8.
  2. Mansharmani GG, Sakuntla R, Bisht DB. Idiopathic Clubbing. Indian J Chest Dis 1970;12:123-30.
  3. Baragwanth P. Idiopathic clubbing. N Engl J Med 2001;344:611.
  4. Spicknall KE, Zirwas MJ, English JC. Clubbing: An update on diagnosis, differential diagnosis, pathophysiology, and clinical relevance J Am Acad Dermatol 2005;52(6):1020-8.
  5. Currie AE, Gallagher PJ. The pathology of clubbing: vascular changes in the nail bed. Br J Dis Chest 1988;82:382-85.
  6. Atkinson S, Fox SB. Vascular endothelia growth factor (VEGF)-A and platelet derived growth factor (PDGF) play a central role in the pathogenesis of digital clubbing. J Pathol 2004;203:721-28 .
  7. Zucker-Franklin D, Philipp CS. Platelet production in the pulmonary capillary bed: New ultrastructural evidence for an old concept. Am J Pathol 2000;157:69-74.
  8. Currie AE, Gallagher PJ. The pathology of clubbing: vascular changes in the nail bed Br J Dis Chest 1988;82:382-85.
  9. Vandemergel X, Renneboog B. Prevalence, aetiologies and significance of clubbing in a department of general internal medicine. Eur J Int Med 2008;19:325-29




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